Management of acquired cholesteatoma in patients with craniofacial anomalies: An institutional experience.

PURPOSE: To analyze the surgical management of cholesteatoma in patients with craniofacial abnormalities, cleft lip/palate by reviewing the institutional experience. The secondary aim was to identify and describe the epidemiological profile of the collected data, and to relate the cleft palate and cholesteatoma. DESIGN AND METHODS: This retrospective chart review includes 97 patients with craniofacial abnormalities and acquired cholesteatoma with anatomopathological proven in 118 ears. The following data were collected from the medical records between 1994 and 2018. RESULTS: The first surgery performed on 76 of the 118 ears (64.4%) was the wall up mastoidectomy, while 42 of the 118 ears (35.5%) received the wall down technique. During the follow-up period of these patients, which ranged from 2 to 29 years, with an average of 13.4 years (±5.88), 77 wall up (40.3%) and 114 wall down (59.6%) mastoidectomies were performed. This brought the total to 191 mastoidectomy surgeries in 118 ears of 97 patients. Of the wall up mastoidectomies, 65 of the 77 (84.4%) presented with cholesteatoma recurrence. In the wall down mastoidectomies follow up, there were new surgical approaches in 15 of the 114 procedures (13.1%), with 6 patients (5.2%) having anatomopathologically proven cholesteatoma recurrences and 9 (7.8%) having clinical instability for cavity cleaning without identification of disease recurrence. CONCLUSIONS: Early approach with wall down/modified techniques guided by specific indication criteria may be more resolute, prevent multiple procedures, and preserve the bone pathway to facilitate possible future hearing rehabilitation in these patients.

[Hearing results after anterior tympanotomy in cholesteatoma surgery].

Between 1987 and 1993, 71 ears of attic type cholesteatoma and 20 ears of adhesive type were operated on using the intact canal wall technique combined with anterior tympanotomy. Pre- and postoperative pure tone hearing after the surgery were analyzed, especially to determine whether anterior tympanotomy is harmful to the inner ear. Mean values of 500, 1000 and 2000Hz of air conduction threshold immediately, before and about 1 year after the operation were compared. In attic cholesteatoma, type 1, type 3, and type 4 of ossiculoplasty revealed significant improvement of 8.4dB, 9.3dB, and 8.0dB respectively. In adhesive cholesteatoma, results obtained were 8.4dB, 0.8dB, and -3.0dB. Differences between pre- and postoperative bone conduction threshold at 4000Hz were 1.5dB in type 1, 1.3dB in type 3, and 5.0dB in type 4 in attic cholesteatoma. Those in adhesive cholesteatoma were -2.5dB, -0.2dB, and -10.0dB respectively. From the results obtained, we concluded that (1) our results were satisfactory with regards to hearing ability following all types of ossiculoplasty in attic cholesteatoma and in type 1 in adhesive cholesteatoma, (2) anterior tympanotomy itself is not a harmful procedure to the inner ear in both types of cholesteatoma, (3) an increase in bone threshold in adhesive cholesteatoma is due to the surgical procedure at the stapes and/or the oval window.

[Familial bilateral atresia of the auditory canal with hypertelorism]. / Familiäre bilaterale Gehörgangsatresie mit Hypertelorismus.

The incidence of malformations of the ear is 1:5000, the incidence of stenosis and atresia is 1:12,000. 30% of the malformations are bilateral. In only 0.3% it is the only malformation. Inherited bilateral atresia of the external auditory canal associated with hypertelorism as the only additional malformation has not yet been described. Basing on a case report the way of interdisciplinary rehabilitation of children affected by bilateral hearing impairment is discussed. Special emphasis is on the discussion of the necessity of radiological examinations for the exclusion of cholesteatoma in an atretic ear which could be found in a 5-year old boy of the described family.